The journal Epilepsia has devoted a Special Issue to “Dravet Syndrome and other Sodium Channel Related Encephalopathies”.  It is Volume 60, Issue S3, and can be accessed online – it contains many good articles, all of which are Open Access. I will point out one here – a review by an international panel of distinguished Dravet syndrome experts, which provides an up-to-date review of treatments, both in an emergency setting and for the longer term.

Most interesting was a proposed protocol for long term treatment evolution. They recommend a first-line treatment with valproate (ex. – Depakote). Once a diagnosis is clear, and if seizures are continuing, a second-line treatment should be added. This could be stiripentol (Diacomit) possibly with clobazam (Onfi).  Other second-line alternatives include topiramate (Topamax), bromide, vagal nerve stimulation, and ketogenic diet. They also make note of two new treatments – cannabidiol is viewed as a possible second-line add-on;  fenfluramine is viewed as a very promising second-line add-on, which could even possibly become a first-line treatment.

The authors make the important point that the treatment landscape is ever-changing, and one must stay ready to adjust any protocol based on new findings.

Cross, JH, et al. Dravet syndrome: Treatment options and management of prolonged seizures. Epilepsia (2019) 60, Suppl 3:S39-S48. DOI: 10.1111/epi.16334